Purpose: To assess macular perfusion in a pediatric population with Wilson disease (WD) using optical coherence tomography angiography (OCTA). Methods: Twenty-six patients diagnosed with WD by conventional laboratory tests and liver biopsy in the pediatric gastroenterology department were enrolled. The superficial (VDs), deep (VDd) and choriocapillary (VDc) vascular densities of the patients were determined, Foveal avascular zones in the superficial (FAZs) and deep (FAZd) layers with central macular thickness (CMT) and subfoveal choroidal thickness (SFCT) were evaluated. OCTA measurements were compared with 35 age- and sex-matched healthy controls. Results: Inferior sector values of VDs, VDd and VDc were significantly increased (P = 0.001, P = 0.002, P = 0.022, resp.). Mean FAZs and FAZd were significantly higher in WD cases (P = 0.001, P = 0.003). No statistical difference was detected in CMT and SFCT values (P > 0.05). Among patients with WD, the nasal sector values of the VDs, VDd and VDc were significantly correlated with 24-hour urinary copper excretion (r = 0.500, P = 0.009; r = 0.608, P = 0.001; r = 0.563, P = 0.003, resp.) and serum caeruloplasmin (r = 0.428, P = 0.029; r = 0.536, P = 0.005; and r = 0.548, P = 0.004, resp.). There was a statistically significant linear correlation between SFCT and hepatic copper level (r = 0.742 and P = 0.015). Conclusions: Alterations of the macular microcirculation were observed in children with WD. OCTA may offer a noninvasive method to evaluate patients with WD when other classic signs may not yet have been presented.